In the 1970’s kidney disease was the most serious complication of scleroderma. It was the leading cause of death and renal dialysis was a common issue for people with the condition. However, scientific advances, the use of anti - hypertensive medication, have all but eliminated this as a problem in scleroderma.
The main problem now affecting people with scleroderma is lung disease, which can be either scarring in the lungs or pulmonary hypertension. The prognosis of these conditions is quite poor and so early recognition to ensure treatment can be initiated and is now a cornerstone of our approach to scleroderma.
The symptoms of possible lung disease include shortness of breath, chest pain with exertion, and cough. It is very important that if someone with scleroderma develops these symptoms that they be taken very seriously and consideration be given to doing further testing.
These tests would include a physical examination by a physician, a chest x-ray, lung function tests and an echocardiogram. If there is evidence of lung scarring and it is caught early there are effective therapies now that can help reduce the inflammation and preserve lung function. This treatment would generally include steroids and other agents that strongly reduce inflammation.
If there is evidence of pulmonary hypertension and disease of the blood vessels, then a number of new therapies have recently improved the outcome. These therapies are designed to help improve blood flow through the lung and include such agents as endothelin antagonists, prostanoids and indeed Viagra, a phosphodiesterase antagonist. These drugs have been shown to significantly improve the symptoms of patients with scleroderma.
It is very important that anyone with scleroderma understands the importance of lung disease and the importance of an early diagnosis. These new treatments are believed to work better the earlier they are used.
So treat your lungs well. It is important that anyone with scleroderma should not smoke cigarettes. They should exercise regularly - staying strong so that they will notice any problems with breathing earlier. You should encourage your doctor to send you for lung function tests every year and make sure that you have an echocardiogram at baseline to evaluate for
pulmonary hypertension.
Dr Sean Gaine
National Pulmonary Hypertension
Mater Misericordiae Hospital